Erdheim–Chester disease: case report with unique postmortem magnetic resonance imaging, high-resolution radiography, and pathologic correlation

Abstract Erdheim–Chester disease is an infiltrative form of histiocytosis characterized by replacement of normal tissues by lipid-laden histiocytes. The disease typically infiltrates the medullary portion of the diaphysis and metaphysis of long bones, producing a characteristic radiological pattern...

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Bibliographic Details
Published in:Clinical imaging 2009-03, Vol.33 (2), p.150-153
Main Authors: de Abreu, Marcelo Rodrigues, Castro, Miguel Oliveira, Chung, Christine, Trudell, Debra, Biswal, Sandip, Wesselly, Michelle, Resnick, Donald
Format: Article
Language:English
Subjects:
Biological and medical sciences
Bone and Bones - diagnostic imaging
Bone and Bones - pathology
Bone histicytosis
Erdheim-Chester Disease - diagnosis
Erdheim-Chester Disease - diagnostic imaging
Erdheim-Chester Disease - pathology
Erdheim–Chester disease
High-resolution X-ray
Humans
Investigative techniques, diagnostic techniques (general aspects)
Magnetic Resonance Imaging
Male
Medical sciences
Middle Aged
Postmortem
Radiography
Radiology
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Summary:Abstract Erdheim–Chester disease is an infiltrative form of histiocytosis characterized by replacement of normal tissues by lipid-laden histiocytes. The disease typically infiltrates the medullary portion of the diaphysis and metaphysis of long bones, producing a characteristic radiological pattern dominated by bone sclerosis. It usually affects adults of 40 years of age with a clinical spectrum ranging from an asymptomatic focal bone lesion to multisystemic disease. This case report documents unique imaging and pathologic findings of Erdheim–Chester disease using close postmortem pathologic-imaging correlation.
ISSN:0899-7071
1873-4499
DOI:10.1016/j.clinimag.2008.09.009